Summary :
Objective: Middle ear salivary gland choristoma are extremly rare. We report a case, describe the clinical management and review the literature. Clinical case: A 12 year old boy presented with unilateral conductive hearing loss associated with a large inferior retraction pocket on otoscopy. CT scan demonstrated a large mass in the left middle ear cavity. The incus was absent and the stapes was partially eroded. Middle ear exploration demonstrated an 8 mm yellow/red mass in the region of the fallopian canal. This mass was comptly removed and histopathology confirmed salivary gland choristoma. Conclusion: These lesions result from an abnormal development of the second branchial arch. It is important to consider these lesions as part of the differential diagnosis for any unilateral hearing loss associated with a middle ear mass in children.